​Kembara Xtra - Medicine - Portal Hypertension

​Kembara Xtra - Medicine - Portal Hypertension 
A rise in portal venous pressure of at least 5 mm Hg that is accompanied with splanchnic vasodilation, the creation of portosystemic collaterals, and hyperdynamic circulation The most frequent cause of this condition is an elevated hepatic venous pressure gradient (HVPG; the difference in pressure between the portal and central venous systems). The condition usually progresses, and there is a chance that it will lead to acute variceal hemorrhage, ascites, hepatic encephalopathy, or hepatorenal syndrome.

Prevention Incidence
Uncertainty surrounds the precise occurrence of portal hypertension.
Prevalence Males outnumber females in terms of predominance of sex, with a prevalence of 200,000 people in the United States. 

Pathophysiology and Etiology 
Causes are typically categorized as follows: - Intrahepatic (most frequently cirrhosis) - Prehepatic (portal vein thrombosis or blockage)
- Posthepatic (right-sided heart failure, Budd-Chiari syndrome, and hepatic vein thrombosis) 90% of intrahepatic instances are brought on by cirrhosis as a result of: - Alcoholism - Virus (hepatitis B, hepatitis C, and hepatitis D)
- Schistosomiasis - Wilson disease - Nonalcoholic fatty liver disease
- Primary biliary cirrhosis (PBC) - Hemochromatosis
and Sarcoidosis
40% of patients with portal hypertension have gastroesophageal variceal formation; increased HVPG causes venous collateral formation in the distal esophagus, proximal stomach, rectum, and umbilicus; and progressing portal hypertension causes splanchnic vasodilation and angiogenesis.
Genetics
No genetic patterns that cause portal hypertension are recognized, with the exception of those linked to certain hepatic disorders.


Child Safety Considerations

The most frequent extrahepatic cause in children is portal vein thrombosis; intrahepatic causes are more frequently biliary atresia, viral hepatitis, and metabolic liver disease.

Prevention 

Patients with cirrhosis may experience less portal hypertension and experience fewer consequences if the underlying liver disease is treated.

Alcoholism, cirrhosis, nonalcoholic fatty liver disease, schistosomiasis, and extrahepatic portal vein thrombosis are associated conditions. 

Diagnoses include: Ascites; chest pain, shortness of breath, and/or edema, which are signs of heart failure; Hematemesis; Melena; Oliguria; Jaundice; Weakness/Fatigue; History of chronic liver disease; Alcoholic hepatitis; Alcohol misuse.

clinical assessment 

Exam results could be generic or particular to certain issues.
Pallor, icterus, digital clubbing, palmar erythema, splenomegaly, caput medusa, spider angiomata, umbilical bruit, hemorrhoids, gynecomastia, and testicular atrophy. Gastroesophageal varices, hypotension, and tachycardia.
Fluid wave, distended abdomen, shifting dullness with percussion, ascites, hepatic encephalopathy, confusion, and coma are some of the symptoms.
- Hyperreflexia - Asterixis

Multiple Diagnoses 
Hemorrhagic gastritis, peptic ulcer disease, Mallory-Weiss tear, gastroesophageal varices with hemorrhage, portal hypertensive gastropathy, peritoneal carcinomatosis, tuberculous peritonitis, hepatocellular carcinoma, fluid overload from heart failure, and nephrotic syndrome are among the conditions that are typically associated with specific presentations.
 Sedative abuse, hepatic encephalopathy, delirium tremens, intracranial hemorrhage, and uremia
Drug-induced nephrotoxicity, renal tubular necrosis, and hepatorenal syndrome

Laboratory Results 

Initial examinations (lab, imaging)
The gold standard for diagnosing portal hypertension is the direct calculation of HVPG (approximation of the gradient in pressure between portal vein and IVC): Free hepatic venous pressure (FHVP) and wedged hepatic venous pressure (WHVP) make up the HVPG. WHVP is calculated by blocking the hepatic vein with a balloon catheter and measuring the blood's proximal static column.
FHVP is calculated with direct measurement of the right atrium, intraabdominal inferior vena cava, or patent hepatic vein.
Esophageal varices typically appear in compensated cirrhosis when HVPG >10 mm Hg and in decompensated cirrhosis when HVPG >16 mm Hg.
 Non-specific modifications linked to underlying illness: Anemia (which may also result from malnutrition or bleeding), leukopenia, and thrombocytopenia are symptoms of hypersplenism. - Liver dysfunction
Hyperbilirubinemia with Hypoalbuminemia
Alkaline phosphatase was increased.
AST and ALT levels that are elevated; abnormal coagulation (prothrombin time, INR, partial thromboplastin time); GI bleeding; iron deficient anemia;
 Increasing serum ammonia
Thrombocytopenia-Hepatorenal syndrome, fecal occult blood,
Elevated blood urea nitrogen (BUN), serum creatinine (Cr), and urine sodium (5 mEq/L; 20 mmol/L)
- Varices, ascites, splenomegaly, and cirrhosis may be found with an MRI, CT scan, or US.
- US/duplex Doppler - Can identify the presence and direction of flow in the portal and hepatic veins - Helpful in identifying ascites or portal vein thrombosis - CT scan/MRI: angiographic assessment of the hepatic venous wedge pressure via the jugular or femoral vein. Correlates with portal pressure.
- The stomach and esophagus may show varices on an upper GI series.
- A noninvasive approach to assess hepatic fibrosis and foretell portal hypertension is transient elastography.

Tests in the Future & Special Considerations
When a patient with no risk factors is diagnosed with portal hypertension, the patient should first be checked for cirrhosis.
The risk of variceal hemorrhage and decompensation is significantly decreased in HVPG response to nonselective -blockers.
Other/Diagnostic Procedures
Serum ascites albumin gradient (SAAG) calculation and diagnostic paracentesis can distinguish between portal hypertension (SAAG >1.1 g/dL) and nonportal hypertensive (SAAG 1.1 g/dL) causes of ascites.
Endoscopy can detect portal hypertensive gastropathy, esophageal and gastric varices, and other conditions.
Specific test interpretation for underlying disease

Management Limit sodium intake since cirrhotic patients readily retain sodium (2 g sodium per day), avoid sedatives that could hasten encephalopathy.

Treatment with medication for encephalopathy.
Initial Line
Prevention of variceal bleeding: - Blockade that is nonselective Nadolol: once-daily oral dose of 20 to 40 mg
Start with 20 to 40 mg PO BID-TID of propranolol.
- As an alternate -blocker, you can think about using carvedilol: 6.25 mg PO once day (1).[C]
- levels may be increased to the highest suggested levels as tolerated; ideal resting heart rate is 55 to 60 bpm. Acute variceal hemorrhage treatment: Pediatric dose: 1 g/kg bolus followed by 1 g/kg/hr is commonly utilized; treat for up to 5 days. - Octreotide: 50 g IV bolus followed by 50 g/hr continuous infusion.
Vasopressin dosage for children should range from 0.002 to 0.005 U/kg/min, with a maximum dose of 0.8 U/min, and should not be higher than 0.01 U/kg/min.
Continue taking the same dose after the bleeding stops for 12 hours, then gradually reduce it over the following 24 to 48 hours.
 To stop a recurrence and to decrease mortality generally: By preventing the adrenergic dilation of the mesenteric arterioles, nadolol (40 to 80 mg/day PO) decreases the amount of portal venous blood that enters the body.
Pediatric dose: 0.5 to 1.0 mg/kg/day PO split q6-8h for propranolol; daily dose: 10 to 60 mg PO BID-QID. - Tetrandrine, a calcium channel blocker, has been demonstrated to lessen negative effects while lowering the rate of rebleeding.
Initial ascites treatment (including salt and fluid restriction): - Furosemide: 20–40 mg/day PO; 1–2 mg/kg/dose PO for children. Albumin IV infusion - Spironolactone: daily oral dose of 50–100 mg; daily oral dose for children: 1–3 mg/kg.
Next Line
Terlipressin is a more selective splanchnic vasoconstrictor and may be linked with fewer side effects (2 mg IV every four hours; titrate down to 1 mg IV every four hours once hemorrhage is under control; may be administered for up to 48 hours). When somatostatin or octreotide therapy fails, it is currently employed.
It has been demonstrated that adding nitrates, such as nitroglycerin or isosorbide mononitrate, lowers mortality and portal pressures and bleeding rates. Nitrates are not regarded as first-line therapy since the risk-benefit ratio is unclear.
Studies on the potential advantages of additional drugs such simvastatin, clonidine, verapamil, and losartan are still being conducted.

Referral 
A primary care physician and a gastroenterologist should work together to manage patients with portal hypertension throughout time.

Surgery is a treatment option for some problems of portal hypertension (in addition to or if medicine is ineffective): - Gastroesophageal varices not accompanied by bleeding
The ideal treatment for preventing bleeding from major (grade III) varices is endoscopic variceal ligation (EVL), which necessitates repeated procedures every 2 to 8 weeks until the varices are completely gone.
- Hemorrhagic gastroesophageal varices
 EVL or sclerosis within 12 hours of manifestation (often the first-line treatment for acute bleeding) (2)[A]
When endoscopic therapy is available, balloon tamponade is infrequently employed. TIPS, or transjugular intrahepatic portosystemic shunt, Portacaval shunting for ascites that is resistant to medical treatment
For patients with advanced disease, large-volume paracentesis, peritoneovenous shunt, TIPS, and liver transplantation should be taken into consideration.

Admission 
Acute GI bleeding should be handled in an inpatient setting, either in the ICU if the patient is unwell or on the regular medical floor if the patient is hemodynamically stable.
Patients who experience encephalopathy-related changes in mental status should be examined in an inpatient environment.
 Initial stabilization and admission requirements - Sudden intestinal bleeding, either through vomiting or the rectum. - If there is acute variceal bleeding: - Acute confusional state or mental status changes
 Cross and type the patient's blood.
 Isotonic fluid should be used for initial resuscitation until packed RBCs are available. Utilize vitamin K and fresh frozen plasma (FFP) to treat coagulopathy.
 Endoscopy immediately following patient stabilization (for both diagnosis and treatment) - Steer clear of sedatives that could hasten encephalopathy.
- Because cirrhotic patients avidly retain salt, limit sodium administration.
- Only restrict protein in encephalopathic patients.
ALERT
If the patient regularly consumes alcohol, test for alcoholic hepatitis and keep an eye out for withdrawal signs and symptoms. Observe inpatient guidelines for managing alcohol withdrawal.
Use isotonic liquid to stay hydrated.
Discharge standards for internal bleeding:
Hemodynamically steady (particularly heart rate) and no active bleeding within 24 hours. For encephalopathy, improvement or resolution of alterations in mental status to baseline. 

Patient Follow-Up Monitoring
Treatment of the chronic liver disease's underlying cause may enhance the structure and function of the liver, which may result in a decrease in the portal pressure gradient.
There is currently no evidence to support the prophylactic use of -blockers in patients without varices.

Diet: Patients with cirrhosis retain sodium; as a result, sodium consumption should be limited; obesity can exacerbate cirrhosis; as a result, diet and exercise should be vigorously advised.
Abstinence from alcohol

Modification of Lifestyle 
Avoid alcohol (see a program if you're dependent), and limit your sodium intake.

Hepatic reserve is assessed using the Child-Pugh classification, which considers encephalopathy, ascites, bilirubin, albumin, and prothrombin. Variceal bleeding: One-third of those with recognized varices will bleed at some point.
- 50% rebleeding, typically within 2 years, barring surgical or TIPS procedures to lower portal pressure.
- Mortality rate of 15-20%
Encephalopathy and ascites frequently return.
Patients with ascites have a poor prognosis, with a 50% 1-year survival rate without liver transplantation (compared to a 90% rate for patients with cirrhosis but no ascites).


Complications include ascites, hepatic encephalopathy, hepatic hydrothorax, hepatorenal syndrome, portal hypertensive gastropathy, portopulmonary hypertension, splenomegaly, and elevated blood pressure (SBP).

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